The impact of heart failure severity at time of listing for cardiac transplantation on survival in pediatric cardiomyopathy

JOURNAL OF HEART AND LUNG TRANSPLANTATION

卷:30

Article

Larsen, Ranae L.²  Canter, Charles E.¹  Naftel, David C.³  Tressler, Margaret³  Rosenthal, David N.⁴  Blume, Elizabeth D.⁵  Mahle, William T.⁶  Yung, Delphine⁷  Morrow, William R.⁸  Orav, E. John⁵  Wilkinson, James D.⁹  Towbin, Jeffrey A.¹⁰  Lipshultz, Stephen E.⁹ 

[1] Washington Univ, Dept Pediat, St Louis, MO 63110 USA

[2] Loma Linda Univ, Dept Pediat, Loma Linda, CA 92350 USA

[3] Univ Alabama, Dept Surg, Birmingham, AL 35294 USA

[4] Stanford Univ, Dept Pediat, Palo Alto, CA 94304 USA

[5] Harvard Univ, Dept Pediat, Dept Internal Med, Boston, MA 02115 USA

[6] Emory Univ, Dept Pediat, Atlanta, GA 30322 USA

[7] Univ Washington, Dept Pediat, Seattle, WA 98195 USA

[8] Univ Arkansas, Dept Pediat, Little Rock, AR 72204 USA

[9] Univ Miami, Dept Pediat, Miami, FL 33152 USA

[10] Univ Cincinnati, Dept Pediat, Cincinnati, OH 45221 USA

关键词: cardiomyopathy;    pediatrics;    heart failure;    mortality;    heart transplantation;   

DOI  :  10.1016/j.healun.2011.01.718

来源: Elsevier

【 摘 要 】

BACKGROUND: The survival benefit of heart transplantation in adult heart failure is greatest for the sickest patients and negligible for patients not requiring inotropic or mechanical support. We hypothesized a similar survival benefit of heart transplantation for childhood cardiomyopathies with heart failure. METHODS: A merged data set of children registered in both the Pediatric Cardiomyopathy Registry and the Pediatric Heart Transplant Study was used to assess differences in mortality before and after transplant in patients with different levels of heart failure severity. Severity was scored 2 if'mechanical ventilatory or circulatory support was required, 1 if intravenous inotropes were required, or 0 if no support was required. RESULTS: For 332 eligible children, 12-month mortality after listing was 9% for those with a severity score of 0 (n = 105), 16% with a score of 1 (n = 118), and 26% with a score of 2 (n = 109; p = 0.002) with a 3%, 8%, and 20% mortality with severity scores at listing of 0, 1, and 2, respectively, occurring before transplant. Patients listed with a score of 0 frequently deteriorated: 50% received an allograft or died before transplant with severity scores of 1 or 2. The risk of deterioration increased with previous surgery (relative risk, 3.84; p = 0.03) in the short-term and with lower left ventricular mass z-score at time of presentation (relative risk, 1.74; p = 0.003) in the longer-term. CONCLUSION: Pediatric cardiomyopathy patients who require high levels of support receive a survival benefit from heart transplantation that is not shared by patients not requiring intravenous inotropic or mechanical support. J Heart Lung Transplant 2011;30:755-60 (C) 2011 International Society for Heart and Lung Transplantation. All rights reserved.

【 授权许可】

Free